Women of all geographic, racial, and ethnic groups are afflicted by autosomal dominant polycystic kidney disease (ADPKD), which is the most common, lethal genetic disease that is inherited as a dominant trait. General issues concerning epidemiology, diagnostic testing, clinical management of pain, hypertension, and intracranial aneurysms are discussed, as well as a short overview of our current understanding of the pathophysiological features, including some new molecular insights. Specific issues affecting the care of women are also discussed, including the management of liver cysts and fertility and pregnancy in ADPKD.